Langerhans cell histiocytosis (LCH) is a rare disease characterised by monoclonal proliferation and infiltration of organs by large mononuclear cells. Organs commonly involved include the lungs and pituitary gland. However, the disease association with hypogonadotrophic hypogonadism has not been reported in the literature, to our knowledge. We report a 26-year-old Chinese man with LCH, recurrent pneumothoraces, diabetes insipidus and hypogonadotrophic hypogonadism. The clinical features and management of the disease are reviewed.